tbuckley/cabot-search huggingface dataset:
CD07 Corticosteroids: a complex case of friend and foe
Authors: Sarah Fleming, Tatiana Galimova, Louise Cunnimgham, Karen Eustace
Journal: British Journal of Dermatology (2023)

Abstract Hypersensitivity reactions to corticosteroids, either immediate or delayed, represent a clinical paradox as one would not expect such reactions to compounds with anti-inflammatory and immunomodulatory effects. We present the case of a 67-year-old patient referred to our service with three episodes of a delayed hypersensitivity reaction to steroids. The first episode occurred in 2016 when the patient was treated for a chest infection with amoxicillin and 5-mg prednisolone. Following 1 day of treatment, the patient developed a widespread erythematous rash. The patient remained systemically well, and the rash resolved over 3 weeks. Subsequently, the patient tolerated penicillin with no issues. In April 2019, following a bupivacaine and triamcinolone steroid injection to the shoulder and back for pain management, the patient developed a widespread erythematous rash. The patient denied any blistering or peeling of the skin and was systemically well. This rash took ∼3 weeks to settle. In August 2019, the patient had a dexamethasone injection to her back and subsequently developed a widespread erythematous pruritic rash. On this occasion, the patient reported symptoms of nausea and headache. The patient has avoided all steroids since 2019 and has developed no rashes. The patient had a negative skin prick test to bupivacaine under the care of immunology. Patch testing results showed positivity to 10/13 compounds tested in the corticosteroid series (Table 1). The patient had a negative patch test result to tixocortol-21-pivalate (0.1%) and to hydrocortisone-21-acetate (1%). In the fragrance series, the patient tested positive to one compound, Santalum album oil (sandalwood oil) 2%. Corticosteroids are classified into four groups (A, B, C and D), with the last subdivided into D1 and D2. A further classification proposed divides corticosteroids into three groups based on their structural characteristics. Allergic reactions to corticosteroids have been reported, with an estimated prevalence of 0.1–0.3%, with contact dermatitis to corticosteroids having a higher frequency (0.5–5%). Although not life-threatening, contact allergy to corticosteroids can have significant impacts on quality of life and on treatment.Table 1Corticosteroid series patch testing resultsHapten/compound%48 h96 hBudesonide0.01+/–+++Betamethasone-17-valerate1++Triamcinolone acetonide1++Tixocortol-21-pivalate0.1Alclometasone-17,21-dipropionate1+/–Clobetasol-17-propionate1++Dexamethasone-21-phosphate disodium salt1+Hydrocortisone-17-butyrate+/–+++Desoximetasone1+++Betamethasone-17,21-dipropionate1+++Methylprednisolone aceponate1+++Corticosteroid mix2.1++++Hydrocortisone-21-acetate1
DP11 Dermal plexiform spindle cell lipoma: a perplexing entity
Authors: Tiarna Byrne, Louise McDonald, Kristofer Holte
Journal: British Journal of Dermatology (2023)

Abstract We present a 66-year-old man who presented to dermatology with an unusual lesion. An outdoor worker with prior history of melanoma, the patient was referred with a new pigmented lesion adjacent to his melanoma scar. This was identified as a seborrhoeic keratosis and the patient was reassured. Following a full body examination, an incidental lesion was identified on the left anterior shoulder. The lesion measured 9 × 7 mm, was dermal-based, dome-shaped, soft to palpation and skin coloured with light peripheral pigmentation. The lesion was featureless upon dermoscopic examination. The clinical differential diagnosis included neurofibroma and an intradermal naevus. The lesion was narrowly excised. Histological examination revealed an ill-defined, wedge-shaped dermal lesion with tumour fascicles with a vaguely plexiform arrangement. The widely separated fascicles comprised loosely arranged spindle cells, mature adipocytes and scant collagen fibres with a myxoid stroma. The spindle cells were stained diffusely with CD34 immunohistochemistry. The adipocytes and a proportion of spindle cells (around 20–30%) were stained with S100. The features were in keeping with a dermal plexiform spindle cell lipoma. Spindle cell lipoma is an uncommon benign, histologically distinct tumour, accounting for 1.5% of all adipocytic neoplasms. More common within the subcutis, it is rare for these lesions to present with a purely intradermal location. Dermal spindle cell lipomas tend to be less cellular than their subcutaneous counterpart. This coupled with the variable representation of the three constituent lesion components (spindle cells, mature adipocytes and fibromucinous stroma) results in a wide range of possible differential diagnoses. The plexiform variant of spindle cell lipoma (PSCL) is a poorly recognized form with even greater potential for misdiagnosis, particularly with plexiform neurofibroma—­leading to an erroneous diagnosis of neurofibromatosis. Dermal PSCL is an exceptionally rare diagnosis with only eight cases reported in the literature thus far. Here, we report a further case of dermal plexiform spindle cell lipoma, with, for the first-time, corresponding clinical images. In our summary, we discuss in detail the potential diagnostic pitfalls of this rare tumour and how to best employ immunohistochemical staining to avoid them.
PA04 (P05) A rare presentation of blistering in an adolescent
Authors: Nicola Watson, Fraser Charlton, Philip Hampton, Anna Dubois, Suzy Leech
Journal: British Journal of Dermatology (2023)

Abstract A previously well 16-year-old male presented with florid and widespread tense bullae and erosions. He gave a 2-week history of an erythematous rash consisting of crusted erosions that started on his chest and face. He denied any new medications or over-the-counter preparations. There was no preceding systemic illness, and no history of cold sores or sexual contacts. In the 24 h prior to his admission, his rash had become widespread, involving his trunk, limbs, hands, ears, face, lips, oral and genital mucosa. He had started to feel systemically unwell and was in significant pain. On examination, he had a combination of heavily crusted erosions and extensive tense bullae containing straw-coloured fluid on a background of inflamed skin. The morphology was herpetiform in some areas. There was notable extension of bullae onto the palms and penis, as well as intraoral and labial erosions. Viral skin swabs were negative, and his biopsy subsequently demonstrated subepidermal blistering with neutrophil-rich inflammation and positive direct immunofluorescence (IMF) for C3 and IgG only. Serum autoantibodies were negative. Childhood bullous pemphigoid (BP) was initially the working diagnosis, but indirect IMF showed localization to the base of the blister and anticollagen VII antibodies later proved positive, consistent with a diagnosis of epidermolysis bullosa acquisita (EBA). Given the clinical features, it was classified as BP-like EBA. He was initially treated with high-dose oral prednisolone, superpotent topical steroids and analgesia as an inpatient. Mycophenolate mofetil (MMF) was initiated, and his corticosteroid dose was slowly tapered. His condition was completely controlled with MMF 500 mg twice daily at 4 months. The patient then elected to stop taking MMF and remains in complete remission at 7 months. The EBA is rare in adults and even more so in children. The mean age of onset is 44–54 years, but up to 5% of reported cases are in children/adolescents. Four subtypes are described: classic mechanobullous, BP-like, mucous membrane pemphigoid-like and linear IgA bullous dermatosis-like. The BP-like EBA is characterized by widespread vesicles and tense bullae, commonly on an inflammatory background and lacking skin fragility, scarring or milia. Oral and genital mucosae are frequently affected in paediatric cases of EBA. Various steroid-sparing treatment modalities are described, including dapsone, colchicine and MMF. The prognosis in the paediatric population is favourable compared with that of adults: the majority achieve complete remission or complete control on treatment.
GD03 Identifying core outcome domains and development of a cutaneous leishmaniasis core outcome measure for use in clinical practice and trials
Authors: Sujai Senarathne, Shalindra Ranasinghe, Indira Kahawita, Vijani Somaratne, Amila Wickramasinghe, Vivak Parkash, Charles Lacey, Paul Kaye, Alison Layton
Journal: British Journal of Dermatology (2023)

Abstract Cutaneous leishmaniasis (CL) is caused by an intracellular protozoan parasite transmitted through a sand fly bite. A review of clinical trials examining different treatment modalities for CL found studies to be deficient in design, execution, analysis and reporting. Core outcome sets (COS), including agreed domains and measures, have been developed for several dermatological disorders. The aims of this study were to reach consensus on the most important core outcome domains to measure when assessing CL, agree on parameters to measure the domains and develop a clinical tool to assess CL. Potential outcome domains for CL were identified through a collaborative international approach involving virtual discussions and e-consultations with stakeholders, including teams caring for CL from Sri Lanka, India, Brazil and the UK. A subsequent judgement process was then undertaken, using the multidisciplinary stakeholder panel of international experts, adhering to nominal group technique to reach final agreement on the core outcome domains. Clinicians presented cases representing the clinical and psychosocial impact of CL to ensure the patient’s perspective was considered. Established core outcome domains included signs and symptoms (objective and subjective), treatment efficacy, treatment impact, clinical sequelae (scarring and pigment) and health-related quality of life (HRQoL). For each core outcome domain, a review of previous approaches to measure the domain was considered. Items to measure the domains were then evaluated by multiple rounds until the panel of experts reached consensus. It was agreed that ‘signs and symptoms’ should capture clinical morphology, diameter and induration of an index lesion with the aid of a palpability score; ‘treatment efficacy and impact’ should capture patient and investigator views through visual analogue scores, percentage change in size of the lesion and re-epithelialization compared with baseline. Sequelae assessment rated pigment, as well as atrophic and hypertrophic/keloid scars. Two open-ended questions were included as a means of capturing some aspects of HRQoL. CutLeishCOM was then generated to capture demographic details, reflect the core outcome domains and assess outcome measurements in each patient. CutLeishCOM has been validated in a small cohort in this study; further validation is required. A specific patient-reported outcome measure should also be considered to ensure adverse effects from treatments, and negative impacts are captured when assessing CL, thus enabling a patient-focused and empathic approach to management.
P04 Leg ulceration in a patient with antisynthetase syndrome
Authors: Xingyue Maria Wang, Julian Pearce, Nilukshi Wijesuriya, Richard Griffiths, Zainab Jiyad
Journal: British Journal of Dermatology (2023)

Abstract A 42-year-old woman presented with leg ulceration on a background of antisynthetase syndrome and immunosuppression with rituximab, azathioprine and prednisolone. The initial lesion was an erythematous patch on the lower leg, which increased in size with violaceous changes. The area subsequently broke down to form an ulcer. On examination, she had a 7-cm ulcerating lesion with central necrosis. Clinically, the appearance was suspicious of pyoderma gangrenosum; biopsy demonstrated scarring without features of vasculitis, atypia or malignancy. The ulcer did not show improvement as expected after oral and topical steroid treatment, with new fleshy nodular growth. Repeat biopsy showed angiocentric atypical lymphocytes with positive Epstein–Barr virus (EBV)-encoded RNA (EBER) staining. Further incisional biopsy demonstrated large, atypical B lymphocytes with a diffuse sheet-like morphology and a poorly circumscribed base. Immunohistochemistry was positive for CD79a, CD30, CD20, MUM1, BCL6, and EBER. Overall, the findings were consistent with EBV-positive diffuse large B-cell lymphoma (DLBCL). The main differential diagnosis was EBV-positive mucocutaneous ulcer (MCU), another EBV-associated lymphoproliferative disorder, albeit with an indolent course (Dojcinov SD, Venkataraman G, Raffeld M et al. EBV positive mucocutaneous ulcer—a study of 26 cases associated with various sources of immunosuppression. Am J Surg Pathol 2010; 34:405–17). In this case, the diffuse sheet-like morphology and poorly circumscribed base favoured EBV-positive DLBCL. The lesion fully healed with withdrawal of immunosuppression and treatment with rituximab therapy, and involved field radiotherapy. First described in 2003, EBV-positive DLBCL is a rare, classically aggressive lymphoma initially believed to only occur in patients older than 50 years of age (Oyama T, Ichimura K, Suzuki R et al. Senile EBV+ B-cell lymphoproliferative disorders. Am J Surg Pathol 2003; 27:16–26). Since the revision of the World Health Organization classification in 2016, it is also recognized to present in younger patients, with an associated better prognosis. EBV-positive DLBCL occurs in both nodal and extranodal sites. Therefore, cutaneous presentations must complete staging investigations to rule out skin involvement secondary to a nodal lymphoma. Our patient highlights a rare complication of immunosuppression. We illustrate the relevance of considering rare causes of ulceration in patients with autoimmune conditions and immunosuppression and demonstrate the importance of considering malignancy in a nonhealing ulcer. Multiple biopsies may be needed in ulcers that poorly respond to treatment. While EBV-positive DLBCL and EBV-positive MCU can be difficult to differentiate, it is vital for prognostication.
P65 Vitamin D deficiency is associated with atopic dermatitis severity in a Bangladeshi population living in the UK
Authors: Rebecca L McCarthy, Bjorn R Thomas, Soha S Tawfik, Ravinder Atkar, Bryan McDonald, Sasha Dhoat, Aaron Hughes, Edel O’Toole
Journal: British Journal of Dermatology (2023)

Abstract Atopic eczema is a common chronic inflammatory skin condition with considerable heterogeneity. South Asian people living in the UK frequently have low vitamin D levels, and those with atopic disease can present with severe eczema. The association between vitamin D insufficiency and eczema severity and the role of vitamin D supplementation in atopic eczema is inconsistent, and it is under-researched in people with Asian ancestry living in low-ultraviolet (UV) areas. This cross-sectional study investigates the association between serum 25-hydroxy vitamin D3 [25(OH)D3] and eczema severity in a cohort of South Asian children and young adults living in the UK. Eligible participants were Bangladeshi children and young adults aged 0–30 years with eczema, participating in the THEA study. Data for the study were collected via parent/patient self-reporting, clinical history and examination and hospital databases. Levels of 25(OH)D3 were documented retrospectively, if available, from hospital databases. Eczema severity was classified by an Eczema Area and Severity Index (EASI) score less than or greater than 10. Multivariate logistic regression was used to adjust for confounding factors. In total, 682 patients were recruited between May 2018 and December 2021. The EASI score was available for 681 participants [mean (SD) 7.9 (9.1)] and 25(OH)D3 results were available for 49.7% (n = 339). Eighty-four per cent were deficient (&amp;lt; 25 nmol L−1) or insufficient (&amp;lt; 50 nmol L−1) in 25(OH)D3. Vitamin D3 levels were inversely correlated with the EASI score (Spearman’s rank R2 = –0.22; P &amp;lt; 0.001). After adjustment, there was a significant association between eczema severity (EASI &amp;gt; 10) and lowest 25(OH)D3 [odds ratio (OR) 0.973, 95% confidence interval (CI) 0.956–0.988; P &amp;lt; 0.001], working class (OR 0.542, 95% CI 0.295–0.991; P = 0.047), previous immunosuppression/biologic therapy (OR 2.432, 95% CI 1.189–5.077; P = 0.016), mild-to-moderate potency topical steroid use on the face and neck (OR 3.566, 95% CI 1.730–7.696; P &amp;lt; 0.001) and topical calcineurin inhibitor use on the face and neck (OR 3.421, 95% CI 1.140–10.288; P = 0.027). These findings demonstrate an inverse correlation between 25(OH)D3 level and eczema severity in a cohort of exclusively South Asian children and young adults living in a low-UV region. Vitamin D plays a role in the modulation of proteins required for skin barrier function and regulation of the innate immune system, suggesting that vitamin D deficiency in this population may contribute to inflammation in the skin. Further research on the role of vitamin D supplementation in atopic eczema, particularly in people with skin of colour, is required.
DP14 Going against the grain: eosinophilic dermatosis presenting as erythema gyratum repens
Authors: Jason Siong Kai Ting, Mark Eisner, Sowmya Venkatesan, Saman Fatah
Journal: British Journal of Dermatology (2023)

Abstract Erythema gyratum repens (EGR) is a rare, paraneoplastic rash with a characteristic woodgrain appearance. It is heavily associated with malignancies, including lung, oesophageal and breast cancers. However, other conditions can mimic EGR, including leucocytoclastic vasculitis, immunobullous and papulosquamous conditions. We report an unusual presentation of eosinophilic dermatosis with an EGR-like pattern, not associated with malignancy. A 67-year-old woman presented with a 2-week history of a pruritic eruption on her legs. It initially started with three small circular erythematous macules on the right leg and one on the left leg. Over the course of a few days, the three macules evolved into large concentric annular patches simulating woodgrain, which raised the clinical suspicion of EGR. After a week, the patches began to blister with accentuation of some of the blisters on the edge of the annular patches. She denied any previous history of skin problems or changes in medication and was otherwise well with no systemic symptoms, including fever. In view of the almost 70% chance of an underlying malignancy, she was thoroughly investigated. Her full blood count, eosinophil count, liver function test, renal profile and serum C3 and C4 were normal. Skin basement membrane/desmosome antibodies and Borrelia burgdorferi IgG and IgM antibodies were also negative. Histology revealed prominent epidermal spongiosis with some corneal and intraepidermal vesicles that were cell-poor and contained only red blood cells. The underlying dermis showed prominent papillary dermal oedema with a brisk interstitial and superficial/deep perivascular mixed inflammatory infiltrate containing numerous eosinophils but no obvious flame figures. There was also eosinophil infiltration into the underlying subcutaneous fat. Direct immunofluorescence was negative. The histological pattern was that of an eosinophil-rich dermatitis with vesiculation; the history and morphology of the eruption were not compatible with bullous insect bites. An eosinophilic dermatosis such as eosinophilic cellulitis presenting with an EGR-like pattern was considered to be the most likely possibility for her presentation. Computed tomography of the neck, thorax, abdomen and pelvis, bidirectional endoscopy and mammography revealed no malignancies. She was treated with prednisolone 30 mg daily for 10 days with Elocon® ointment, and the eruption improved remarkably leaving postinflammatory colour changes that subsequently resolved. We would like to raise the awareness that an EGR-like pattern can be found in several other dermatoses, including eosinophilic dermatosis, and woodgrain pattern is not always equivalent to EGR or a high risk of associated malignancies (Table 1).Table 1Erythema gyratum repens-like dermatosesLeucocytoclastic vasculitisImmunobullousPapulosquamousBullous pemphigoidPsoriasisEpidermolysis bullosa acquisitaPityriasis rubra pilarisLinear IgA dermatoses
P47 Management of extramammary paget disease: a current unmet need
Authors: Sofia Labbouz, Hemalatha Bhuvanai Sitaraaman, Laura Keeling, Ruth Murphy
Journal: British Journal of Dermatology (2023)

Abstract Extramammary Paget disease (EMPD) is a rare epithelial malignancy predominantly affecting the anogenital skin. Primary EMPD carries a risk of invasion and metastasis, as well as malignancy in contiguous organs. No UK guidelines for the management of EMPD exist, resulting in a lack of consensus for staging and screening for associated cancers. We collected consecutive cases of histologically diagnosed EMPD at a regional centre (2017–2022). The aim was to characterize disease presentation, staging and treatment. We identified 22 patients [12 (55%) women, 10 (45%) men; mean age 72 years]. Affected sites were the vulva (n = 10; 45%), male genitalia (n = 7; 32%), perianal area/buttocks (n = 3; 14%), suprapubic area (n = 2; 9%) and axilla (n = 1; 4%). One patient had vulval and anal EMPD. All patients were referred from primary care to multiple secondary care services, including dermatology (n = 9; 41%), gynaecology (n = 9; 41%), urology (n = 2; 9%) and colorectal surgery (n = 1; 4.5%). Nine patients (41%) were jointly managed with dermatology. Sixteen (73%) patients were discussed at a multidisciplinary team (MDT) meeting; nine (41%) at the specialist skin cancer MDT and seven (32%) at other MDTs (anal cancer, colorectal, urology, oncology, gynae-oncology). Screening for associated malignancy was performed for 14 patients (64%). Eleven patients (50%) had histological evidence of invasion of their primary EMPD, of which nine (82%) underwent advanced imaging with computed tomography, positron emission tomography or magnetic resonance imaging. Treatment was directed by the stage of the disease (Table 1). Three patients (14%) developed metastatic EMPD and died despite radiochemotherapy or chemotherapy and surgery. Our data confirm a low prevalence of EMPD, but mortality for metastatic EMPD appears high. EMPD is a complex disease involving the skin. Patients are best managed by a specialist skin cancer MDT in conjunction with other relevant cancer MDTs. A greater understanding of this disease is needed to standardize care. Table 1Treatment received by our patient cohort with corresponding staging of extramammary Paget disease (EMPD)TreatmentPatients, n (%)Tumour classificationEMPD stagingTopical agents5 (23)TisIntraepithelial (in situ) EMPDSurgery12 (54)T1Tumour thickness ≤ 4 mm and no lymphovascular invasionChemotherapy/radiotherapy ± surgery5 (23)T2Tumour thickness &amp;gt; 4 mm or lymphovascular invasion